Acute rheumatic fever (ARF) is a disease
characterized by a systemic inflammatory response that occurs approximately
three weeks after an untreated group A beta-haemolytic streptococcal pharyngeal
infection. Though the incidence of ARF in the developed world is on the
decline, the same is not true of developing nations like Nigeria. Appropriate
antibiotic therapy during pharyngeal infection essentially eliminates the
future risk of developing rheumatic fever [1].

Diagnosis of ARF is clinical, using
the revised Jones criteria of 1992 [2]. Evidence of two
major criteria or one major criterion and at least two minor criteria is enough
to make a diagnosis. The major criteria include pancarditis, polyarthritis,
chorea, erythema marginatum and subcutaneous nodules. The minor criteria
include athralgia, fever, elevated erythrocyte sedimentation rate (ESR),
increased C-reactive protein and first degree atrio-ventricular block [1]. The most feared consequence of the pathologic processes is
chronic scarring leading to valvular stenosis and regurgitations. Treatment may
involve the use of aspirin and/or corticosteroids. Prevention of recurrence of
carditis and ARF involves long-term administration of benzathine penicillin.

Case report

O.T, a 20-year old Nigerian female
student was seen in July 2007 with history of recurrent breathlessness which
had started about 10 years earlier. Her symptoms worsened in the previous three
weeks when she became breathless at rest, had paroxysmal nocturnal dyspnoea,
bilateral leg swelling and cough productive of whitish, and blood-stained
sputum. Past history was significant for recurrent sore throat in childhood and
skin rashes. She was not previously diagnosed hypertensive or asthmatic. She
was the second child in a monogamous family; both parents are in a very low
income group. Her genotype was AS and she was not smoking cigarettes or
consuming alcohol. There was no family history of the disease in any of her
siblings.

Examination revealed that the woman
looked pale, was chronically ill, and had a tinge of jaundice with severe
bilateral pitting pedal oedema. A cardiovascular system examination revealed a
pulse rate of 120 per minute, irregular and small volume, blood pressure of
90/60mmHg, elevated jugular venous pressure, displaced apex beat which was
located at 7th intercostal space anterior axillary line and a fourth, first and
second heart sounds. The respiratory rate was 36 cycles per minute and
bilateral crepitations. Other examination findings were a tender, pulsatile
hepatomegaly of 6 cm below the right coastal margin.

An echocardiography showed densely
thickened mitral valves with severe commissural fusion leading to doming of the
mitral valve in diastole, markedly dilated left atrium with an intramural clot
attached to the posterior left atrial wall, reduced left ventricular ejection
fraction, markedly dilated right atrium and right ventricle. Colour flow showed
severe tricuspid regurgitation. The calculated mitral valve area is 0.67cm
square. Complete blood count showed leucocytosis with a white cell
count of 19,700/cubic centimetres and marked
neutrophilia. The chest X-ray revealed cardiomegaly with a double cardiac
shadow in keeping with the dilated left and right atrium (Figure 1).

She was placed on diuretics
(frusemide and low-dose spironolactone) Angiotensin
Converting Enzyme inhibitor (Lisinopril), intranasal oxygen, digoxin,
subcutaneous clexane and antibiotics.On the fifth day of admission, she
suddenly deteriorated and became restless and breathless at rest, at which time
the pulse and the blood pressure were not recordable. She thereafter died. The
relatives did not consent to autopsy.

Discussion

Rheumatic fever, no doubt remains a
disease with great morbidity and mortality in most low and middle income
countries despite having been almost eradicated in high income countries [1]. High frequency and severity of rheumatic heart disease is
still been reported in many parts of Africa [2,3].

Rheumatic fever (rheumatic heart
disease) is likely to have developed as a non-suppurative complication of group
A- beta haemolytic streptococcal pharyngitis due to delayed immune response [4], as it is found in the case presented here. An estimated 1%
of all school children in developing countries shows signs of rheumatic heart
disease [5].

Epidemiological association between
group A beta haemolytic streptococcal throat infection and the subsequent
development of acute rheumatic fever in Nigerians have been well established [6]. Sani et al [7], showed that rheumatic
heart disease is still a major cause of morbidity among Nigerians and that many
already had complications at presentation. There have also been concerns about
the possibilities of the non-group A beta haemolytic streptococci to cause
rheumatic fever and acute glomerulonephritis [4,5,8].

The case being presented typifies
the cost of late presentation in patients with rheumatic heart disease. The cause
of death in the case was likely to have been pulmonary thromboembolism due to
the intracardiac thrombus and/ or deep venous thrombosis seen on
echocardiography and the sudden nature of the mortality. Effective screening
and treatment at the early stages are more likely to reduce the burden of the
disease. Management of rheumatic heart disease involves a great deal of
manpower, skills, facilities and financial resources which are very limted in
Nigeria [9].

Therefore, prevention at all levels
should be aggressively undertaken to reduce to the barest minimum if not
eradicate the morbidities and mortalities associated with rheumatic
fever/rheumatic heart diseases in Nigeria and other countries in Africa [9].

The Drakenberg declaration on the
control of rheumatic fever and rheumatic heart disease in Africa was released
from the First Pan African conference of Rheumatic fever and rheumatic heart
disease held in South Africa in 2005. It highlighted programmes which include
effort to increase awareness of rheumatic fever and rheumatic heart disease
among general public and practitioners. This includes the establishment of
surveillance programmes to measure the burden of disease in the population, advocacy
to increase allocation of resources for the treatment of affected children and
young adults, and the implementation of primary and secondary prevention
schemes in all countries of Africa [4,10].

Similar reports have emanated from
other centres highlighting the burden of rheumatic heart disease [2,9]. Many of the patients come to the
hospital when surgery is not a feasible option as in this case with severe
pulmonary hypertension. Others that are fortunate to be detected much earlier
had to wait for support from philanthropists and organizations for financial
support to embark on the overseas journey for the surgical repair. Many die
from various complications such as cardiac arrhythmias, thromboembolism, while
waiting. Tackling poverty in developing countries therefore remains paramount
in eradicating this disease if the millennium development goals must be
achieved.

Conclusion

Rheumatic heart disease remains a
disease that is still ravaging the developing economy of the world and
paradoxically where resources are scarce to control it. Therefore, prevention
and appropriate treatment of rheumatic fever and all febrile illness in
childhood is very crucial to eradicating this disease.

Conflicts of interests

Tha authors declare they have no conflicts
of interests.

Figures

Figure 1: Postero-anterior view of Chest X-ray of the
patient. There is massive cardiomegaly and upper lobe blood diversion.

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